DPH5

Chr 1AR

diphthamide biosynthesis 5

Also known as: AD-018, CGI-30, HSPC143, NEDSFF, NPD015

This gene encodes a component of the diphthamide synthesis pathway. Diphthamide is a post-translationally modified histidine residue found only on translation elongation factor 2. It is conserved from archaebacteria to humans, and is targeted by diphtheria toxin and Pseudomonas exotoxin A to halt cellular protein synthesis. The yeast and Chinese hamster homologs of this protein catalyze the trimethylation of the histidine residue on elongation factor 2, resulting in a diphthine moiety that is subsequently amidated to yield diphthamide. Multiple transcript variants encoding different isoforms have been found for this gene. [provided by RefSeq, Jul 2008]

OMIMResearchGenerating clinical summary…

Primary Disease Associations & Inheritance

Neurodevelopmental disorder with short stature, prominent forehead, and feeding difficultiesMIM #620070
AR

Clinical highlights

Gene-disease validity (ClinGen)
neurodevelopmental disorder with short stature, prominent forehead, and feeding difficulties · ARLimitednot for standalone diagnostic reporting
0
Active trials
2
Pubs (1 yr)
P/LP submissions
P/LP missense
1.27
LOEUF
DN
Mechanism· predicted

Population Genetics & Constraint

gnomAD v4 — loss-of-function & missense intolerance

Tolerant — LoF & missense variants common in population
LoF Constraint?
1.27LOEUF
pLI 0.000
Z-score 0.82
OE 0.77 (0.481.27)
Tolerant

Highly tolerant — LoF variants common in population

Missense Constraint?
0.48Z-score
OE missense 0.89 (0.771.03)
131 obs / 147.4 exp
Tolerant

Mild missense constraint

Observed / Expected Ratios?
LoF OE?0.77 (0.481.27)
00.351.4
Missense OE?0.89 (0.771.03)
00.61.4
Synonymous OE?1.06
01.21.6
LoF obs/exp: 11 / 14.3Missense obs/exp: 131 / 147.4Syn Z: -0.36

ClinVar Variant Classifications

0 submitted variants in ClinVar

Protein Context — Lollipop Plot

DPH5 · protein map & ClinVar variants

Showing all ClinVar variants across the protein. Search a specific variant to highlight its position.

Clinical Trials

Active and recruiting trials from ClinicalTrials.gov

No active trials found for this gene.

Search ClinicalTrials.gov →